Kasabach-Merritt syndrome

Kasabach-Merritt syndrome, also known as haemangioma thrombocytopenia syndrome, is a rare life-threatening disease found in infants in which a rapidly growing vascular tumour is responsible for thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy.

Vascular tumours may grow very rapidly, which can induce severe thrombocytopenia and severe consumptive coagulopathy. In severe cases, there may be disseminated intravascular coagulation.

Kasabach-Merritt syndrome is usually caused by kaposiform haemangioendothelioma or tufted angioma. Other vascular tumours may be responsible, such as infantile haemangioma.

The radiographic features are specific to each vascular tumour. They can be found in multiple locations, more frequently subcutaneous, musculoskeletal, splenic and hepatic in location.

Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome - systemic corticosteroids, radiation therapy, compression, embolisation and/or pharmacotherapy (e.g. interferon, platelet aggregation inhibitors) 4.

Repetitive haemarthrosis can also occur, leading to a haemophilia-like arthropathy 1. Other complications include congestive heart failure and gastrointestinal bleeding 3.

The syndrome is named after Haig Haigouni Kasabach and Katharine Krom Merritt, the two paediatricians who first described the condition in 1940.

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Article information

rID: 1549
Section: Syndromes
Synonyms or Alternate Spellings:
  • Kasabach Merritt syndrome
  • Kasabach and Merritt syndrome
  • Haemangioma thrombocytopenia syndrome
  • Haemangioma thrombocytopaenia syndrome
  • Hemangioma thrombocytopenia syndrome

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